nogrЭкспериментальная и клиническая гастроэнтерологияExperimental and Clinical Gastroenterology1682-8658«Global Media Technologies»10.31146/1682-8658-ecg-196-12-154-172nogr-1813Research ArticleКЛИНИЧЕСКИЕ РЕКОМЕНДАЦИИCLINICAL GUIDELINESРоссийский консенсус «Гипераммониемии у взрослых» (Версия 2021)Russian Consensus on “Hyperammonemia in Adults”: The 2021 Version (in English)ЛазебникЛ. Б.LazebnikL. B.

127473, Москва, ул. Делегатская, 20/1

127473, Moscow, Delegatskaya St., 20, p. 1

Leonid.BorisL@gmail.comГоловановаЕ. В.GolovanovaE. V.

127473, Москва, ул. Делегатская, 20/1

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680000, Дальневосточный федеральный округ, Хабаровский край, г. Хабаровск, ул. Муравьева-Амурского, 35,

680000, Far Eastern Federal District, Khabarovsk Territory, Khabarovsk, st. Muravyov-Amursky, 35

БуеверовА. О.BueverovA. O.

119991, г. Москва, ул. Трубецкая, д. 8, стр. 2 129110, г. Москва, ул. Щепкина 61/2

119991, Moscow, Trubetskaya St., 8 building 2  129110, Moscow

ПлотниковаЕ. Ю.PlotnikovaE. Y.

650056, г. Кемерово, ул. Ворошилова, 22А

650056, Kemerovo, Voroshilov street, 22A

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454092, Челябинская область, г. Челябинск, улица Воровского, 64

454092, Chelyabinsk, Vorovskogo str., 64

ИльченкоЛ. Ю.IlchenkoL. Y.

117997, Москва, ул. Островитянова д. 1

117997, Moscow, Ostrovityanova St., 1

ЕрмоловаТ. В.ErmolovaT. V.

191015, Санкт-Петербург, Кирочная ул., 41

191015, St. Petersburg, Kirochnaya street, 41

ТарасоваЛ. В.TarasovaL. V.

628412, Ханты-Мансийский автономный Округ-Югра, г. Сургут, пр. Ленина, 1428015, Московский просп., Чебоксары, 15

628412 Khanty-Mansi Autonomous Region—Ugra, Surgut, Lenina street, 1428015, Cheboksary, Moskosvsky Pr., 15  

ЛиЕ. Д.LeeE. D.

117647, Москва, Севастопольский пр., 66

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ЦыгановаЮ. В.TsyganovaY. V.

428015, Московский просп., Чебоксары, 15

428015, Cheboksary, Moskosvsky Pr., 15 

АхмедовВ. А.AkhmedovV. A.

644099, г. Омск, ул. Ленина, 12

644099, Omsk, Lenin str., 12

АгееваЕ. А.AgeevaE. A.

680000, г. Хабаровск, ул. Дикопольцева, 34

680000, Khabarovsk, st. Dikopoltseva, 34

ЛосевВ. М.LosevV. M.

443099, г. Самара, ул. Чапаевская, 89

443099, Samara, Chapayevskaya St., 89  

КуприяноваИ. Н.KupriyanovaI. N.

620219, г. Екатеринбург, ул. Репина, 3 

620219, Еkaterinburg, Repina St., 3

СериковаС. Н.SerikovaS. N.

350012, г. Краснодар, ул. Красных Партизан, 6/2

350012, Krasnodar, st. Red Partisans, 6/2

КорочанскаяН. В.KorochanskayaN. V.

350012, г. Краснодар, ул. Красных Партизан, 6/2

350012, Krasnodar, st. Red Partisans, 6/2

ВологжанинаЛ. Г.VologzhaninaL. G.

614990, г. Пермь, ул. Петропавловская, д. 26

614099, Perm Region, Perm, Petropavlovskaya St., 26

ЦиммерманЯ. С.ZimmermanY. S.

614990, г. Пермь, ул. Петропавловская, д. 26

614099, Perm Region, Perm, Petropavlovskaya St., 26

СасЕ. И.SasE. I.

194044, Санкт-Петербург, улица Академика Лебедева, д. 6

194044, St. Petersburg, Ak. Lebedev st., 6

ЖуравельС. В.ZhuravelS. V.

127473, Москва, ул. Делегатская, 20/1129090, г. Москва, Б. Сухаревская пл., д. 3

127473, Moscow, Delegatskaya St., 20, p. 1Moscow, 129090, B. Sukharevskaya sq., 3

ОковитыйС. В.OkovitiyS. V.

197376, Санкт-Петербург, ул. Профессора Попова д. 4

197376, St. Petersburg, st. Professor Popov 4

ОсипенкоМ. Ф.OsipenkoM. F.

630091, г. Новосибирск, Красный проспект, 52

630091, Novosibirsk, Krasnyj prospect, 52

РадченкоВ. Г.RadchenkoV. G.

194044, Санкт-Петербург, улица Академика Лебедева, д. 6

194044, St. Petersburg, Ak. Lebedev st., 6

СолдатоваГ. С.SoldatovaG. S.

630090, Новосибирская область, г. Новосибирск, ул. Пирогова, д. 1

630090, Novosibirsk, Pirogova str., 1

СиткинС. И.SitkinS. I.

191015, Санкт-Петербург, Кирочная ул., 41197110, Санкт-Петербург, Пудожская ул., д. 7

191015, St. Petersburg, Kirochnaya street, 41197110, St. Petersburg, Pudozhskaya st., 7

СеливерстовП. В.SeliverstovP. V.

191015, Санкт-Петербург, Кирочная ул., 41

191015, St. Petersburg, Kirochnaya street, 41

ШавкутаГ. В.ShavkutaG. V.

344022, г. Ростов-на-Дону, пер. Нахичеванский, 29

344022, Rostov-on-Don, Nakhichevansky Lane, 29

БутоваЕ. Н.ButovaE. N.

344022, г. Ростов-на-Дону, пер. Нахичеванский, 29

344022, Rostov-on-Don, Nakhichevansky Lane, 29

КожевниковаС. А.KozhevnikovaS. A.

394036, Воронежская область, г. Воронеж, улица Студенческая, д. 10

394036, Voronezh, Studencheskaya Street, 12

ФГБОУ ВО «Московский государственный медико-стоматологический университет им. А. И. Евдокимова» Минздрава РоссииРоссия“Moscow State University of Medicine and Density n. a. A. I. Evdokimov”Russian FederationФГБОУ ВО ДВГМУ Минздрава РоссииРоссияThe Far Eastern State Medical UniversityRussian FederationФГАОУ ВО Первый МГМУ им. И. М. Сеченова Минздрава России; ГБУЗ МО МОНИКИ им. М. Ф. ВладимирскогоРоссияI. M. Sechenov first Moscow state medical university (Sechenov university); Moscow regional research and clinical Institute of M. F. VladimirskyRussian FederationФедеральное Государственное Бюджетное Учреждение Высшего Образования Кемеровский государственный медицинский университет Минздрава РоссииРоссияFederal State Budgetary Institution of Higher Education Kemerovo state medical University of the Ministry of health of RussiaRussian FederationФГБОУ ВО «Южно-Уральский государственный медицинский университет» Минздрава РоссииРоссия“South-Ural State Medical University” of the Ministry of Health of RussiaRussian FederationФГБОУ ВО Российский национальный исследовательский медицинский университет имени Н. И. Пирогова Минздрава РоссииРоссияPirogov Russian National Research Medical University (RNRMU)Russian FederationФедеральное государственное бюджетное образовательное учреждение высшего образования «Северо-Западный государственный медицинский университет им. И. И. Мечникова» Министерства здравоохранения Российской ФедерацииРоссияNorth-Western state medical University named after I. I. Mechnikov, Ministry of health of the Russian FederationRussian FederationБУ ВО ХМАО-Югры «Сургутский государственный университет»; ФГБУ ВО «Чувашский государственный университет» Минздрава РоссииРоссияBI of HE “The Surgut State University”; The Chuvashian State UniversityRussian FederationМногопрофильный медицинский центр Банка РоссииРоссияMultifocal Medicine Center of The Central Bank of Russian FederationRussian FederationФГБУ ВО «Чувашский государственный университет» Минздрава РоссииРоссияThe Chuvashian State UniversityRussian FederationФГБОУ ВО «Омский государственный медицинский университет» Минздрава РоссииРоссия“Omsk State Medical University” of the Ministry of HealthRussian FederationКГБУЗ «Городская клиническая поликлиника No 3» МЗХКРоссияKHSBI “City Clinical Polyclinic No 3”Russian FederationСамарский государственный медицинский университетРоссияSamara State Medical UniversityRussian FederationФГБОУ ВО «Уральский государственный медицинский университет» Минздрава РоссииРоссия“Ural state medical University” of the Ministry of health of the Russian Federation, Department of Polyclinic therapy, ultrasound and functional diagnosticsRussian FederationГосударственное бюджетное учреждение здравоохранения «Краевая клиническая больница No 2» Министерства здравоохранения Краснодарского краяРоссияState Budgetary Institution of Health Care “Region Clinic Hospital Nr 2” Health Ministry of Krasnodar RegionRussian FederationФГБОУ ВО «Пермский государственный медицинский университет имени академика Е. 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Sklifosovskiy of Healthcare Department of MoscowRussian FederationСанкт-Петербургский государственный химико-фармацевтический университет (СПХФУ)РоссияSaint Petersburg State Chemical Pharmaceutical University (SPCPA)Russian FederationФГБОУ ВО Новосибирский государственный медицинский университет Минздрава РоссииРоссияPublic budgetary educational institution of higher education “Novosibirsk State Medical University” of the Ministry of Healthcare of the RussiaRussian FederationНовосибирский ГУ, АкадемгородокРоссияNovosibirsk State University, AkademgorodokRussian FederationФедеральное государственное бюджетное образовательное учреждение высшего образования «Северо-Западный государственный медицинский университет им. И. И. Мечникова» Министерства здравоохранения Российской Федерации; ФГУП «Гос. НИИ ОЧБ» ФМБА РоссииРоссияNorth-Western state medical University named after I. I. Mechnikov, Ministry of health of the Russian Federation; Federal Medical and Biological Agency “State Research Institute of Highly Pure Biopreparations”Russian FederationФГБОУ ВО РостГМУ Минздрава РоссииРоссияRostov State Medical University of the Ministry of Health of RussiaRussian FederationФГБОУ ВО «ВГМУ им. Н. Н. Бурденко» Минздрава РоссииРоссияVoronezh State Medical University named after N. N. BurdenkoRussian Federation202116012022012154172Copyright © Лазебник Л.Б., Голованова Е.В., Алексеенко С.А., Буеверов А.О., Плотникова Е.Ю., Долгушина А.И., Ильченко Л.Ю., Ермолова Т.В., Тарасова Л.В., Ли Е.Д., Цыганова Ю.В., Ахмедов В.А., Агеева Е.А., Лосев В.М., Куприянова И.Н., Серикова С.Н., Корочанская Н.В., Вологжанина Л.Г., Циммерман Я.С., Сас Е.И., Журавель С.В., Оковитый С.В., Осипенко М.Ф., Радченко В.Г., Солдатова Г.С., Ситкин С.И., Селиверстов П.В., Шавкута Г.В., Бутова Е.Н., Кожевникова С.А., 20222022Лазебник Л.Б., Голованова Е.В., Алексеенко С.А., Буеверов А.О., Плотникова Е.Ю., Долгушина А.И., Ильченко Л.Ю., Ермолова Т.В., Тарасова Л.В., Ли Е.Д., Цыганова Ю.В., Ахмедов В.А., Агеева Е.А., Лосев В.М., Куприянова И.Н., Серикова С.Н., Корочанская Н.В., Вологжанина Л.Г., Циммерман Я.С., Сас Е.И., Журавель С.В., Оковитый С.В., Осипенко М.Ф., Радченко В.Г., Солдатова Г.С., Ситкин С.И., Селиверстов П.В., Шавкута Г.В., Бутова Е.Н., Кожевникова С.А.Lazebnik L.B., Golovanova E.V., Alekseenko S.A., Bueverov A.O., Plotnikova E.Y., Dolgushina A.I., Ilchenko L.Y., Ermolova T.V., Tarasova L.V., Lee E.D., Tsyganova Y.V., Akhmedov V.A., Ageeva E.A., Losev V.M., Kupriyanova I.N., Serikova S.N., Korochanskaya N.V., Vologzhanina L.G., Zimmerman Y.S., Sas E.I., Zhuravel S.V., Okovitiy S.V., Osipenko M.F., Radchenko V.G., Soldatova G.S., Sitkin S.I., Seliverstov P.V., Shavkuta G.V., Butova E.N., Kozhevnikova S.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.nogr.org/jour/article/view/1813

Гипераммонемия — это острая или хроническая интоксикация аммиаком и аммонием, связанная с повышенным уровнем аммиака в сыворотке крови из-за его повышенной выработки и/или снижения детоксикации. Гипераммонемия может быть результатом множества причин и клинически проявляться неспецифическими признаками и симптомами, включая астению, энцефалопатию, стеатоз или фиброз печени и саркопению. При нарушении функции печени гипераммониемия чаще всего проявляется (микро) энцефалопатией. Таким образом, в случае неожиданного изменения психического статуса необходимо как можно быстрее исключить гипераммониемию. Экспресс-метод фотометрического анализа достаточно информативен для определения уровня аммиака. Предлагается следующая классификация гипераммонемии: а) по уровням аммиака (нормальный уровень: ≤ 60 мкмоль / л; легкая (степень 1): ≤ 100 мкмоль / л; средняя (степень 2): ≤ 200 мкмоль / л; и тяжелая (степень 3):> 200 мкмоль / л); б) по этиопатогенезу (наследственный (врожденный), функциональный (физиологический), приобретенный (печеночный, внепеченочный, смешанный)); в) по клинической картине (преходящая, рецидивирующая или стойкая, постоянная (стабильная, без лечения), скрытая). Лечение гипераммониемии направлено на лечение основного заболевания и включает диету, ограниченную животным белком, но содержащую достаточное количество растительного белка, ограниченную физическую активность и использование кишечных невсасывающихся антибиотиков (рифаксимин-альфа), а также пре- и пробиотиков. … L-орнитин- L-аспартат (LOLA) — это базовый терапевтический продукт, применяемый в ряде сценариев для коррекции уровня гипераммониемии.

Hyperammonemia is an acute or chronic intoxication with ammonia and ammonium associated with elevated ammonia levels in serum due to either its increased production and/or decreased detoxification. Hyperammonemia can result from a variety of causes and clinically presents with unspecific signs and symptoms, including asthenia, encephalopathy, liver steatosis or fibrosis, and sarcopenia. With impaired liver function, hyperammonemia most frequently manifests in (micro)encephalopathy. Thus in case of unexpect change in mental status hyperammonemia must be excluded as fast as possible. An express method of photometric assay is informative enough to determine the ammonia levels. The following hyperammonemia classification is proposed: a) by ammonia levels (normal level: ≤ 60 μmol/L; mild (Grade 1): ≤ 100 μmol/L; moderate (Grade 2): ≤ 200 μmol/L; and severe (Grade 3): > 200 μmol/L); b) by etiopathogenesis (hereditary (congenital), functional (physiological), acquired (hepatic, extrahepatic, mixed)); c) by clinical presentation (transient, recurrent or persistent, constant (stable, without treatment), covert). Treatment for hyperammonemia is aimed at treating the primary disease and includes a diet that is restricted in animal protein but contains sufficient vegetable protein, limited physical activities, and use of intestinal non-absorbable antibiotics (rifaximin- alpha) as well as pre- and probiotics. L-ornithine- L-aspartate (LOLA) is a baseline therapeutic product administered in a number of scenarios to correct the level of hyperammonemia.

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The authors declare that there are no conflicts of interest present.